Presentation and management of pulmonary artery sarcoma

Abstract

BackgroundPulmonary artery sarcoma (PAS) is a rare but aggressive malignancy that leads to heart failure and death without treatment. Here we reviewed the presentation and management of patients treated at a national centre for pulmonary endarterectomy (PEA) and its associated hospital in Cambridge, UK.MethodsDetails of PAS patients treated at Papworth and Addenbrooke’s Hospitals between 2000 and 2014 were reviewed.ResultsTwenty patients were diagnosed with PAS (11 males, 9 females), with a median age of presentation of 57 years (range 27–77). Presenting symptoms include dyspnoea (20), chest pain/tightness (7), oedema (5), constitutional symptoms (5), cough (3) and haemoptysis (3). Twelve patients were in group III/IV of the NYHA functional classification of symptoms. Initial CT scans were suggestive of thromboembolism in seven patients. Histological findings were of intimal sarcoma (13) and high grade sarcoma NOS (6).Median overall survival (OS) was 17 months. Fourteen patients underwent PEA to relieve vascular obstruction, while six had inoperable and/or metastatic disease. There were three peri-operative deaths. Although there was no difference in median OS between patients who had PEA and those who did not (20 vs 17 months, P = 0.2488), surgery provided significant symptomatic improvement and some with long-term survival. Five patients received post-surgical chemotherapy (anthracycline +/− ifosfamide), and after completion four also had radiotherapy. Patients who received post-operative chemo- and radio-therapy showed a trend towards better survival compared to those who had surgery alone (24 vs 8 months, P = 0.3417). For palliative chemotherapy, partial responses were observed with the VID regimen and pegylated liposomal doxorubicin. Stable disease was achieved in a patient with intimal sarcoma with rhabdomyosarcomatous differentiation on third-line cisplatin and topotecan. The longest surviving patient (102 months) has had PEA, adjuvant epirubicin and radiotherapy. She developed lung metastases 7 years later, which were treated with radiofrequency ablation.ConclusionsPAS often presents with symptoms mimicking pulmonary hypertension, heart failure or thromboembolic disease. PEA provides good symptomatic relief and in some cases, offers a chance of long-term survival. Although outcome appears to be better when PEA is combined with post-operative chemo- and radio-therapy, further studies are warranted.

A Case of Unresectable Pulmonary Artery Intimal Sarcoma with Prolonged Survival by Chemotherapy

“…However, the serial chemotherapeutic regimens used in our case may have contributed to her prolonged survival of 14.2 months. This survival period was comparable to the results reported in the literatures, such as 8–17 months in patients with advanced and metastatic pulmonary intimal artery sarcoma [7, 8]. …”

“…The prognosis of unresectable or metastatic disease has historically been extremely poor, with median survival of 1.5 months three decades ago [6]. However, due to advances in chemotherapy and radiotherapy, the survival of such patients with advanced pulmonary artery intimal sarcoma has improved markedly, ranging from 8 to 17 months [7, 8]. Doxorubicin-based chemotherapeutic regimens are commonly used in patients with unresectable pulmonary artery intimal sarcoma.…”

Pulmonary artery intimal sarcoma misdiagnosed as pulmonary embolism: A case report

“…However, another study reports that FDP-PET has been useful for diagnosis (17). Intimal sarcoma of the pulmonary artery has no specific symptoms at an early stage and has been easily misdiagnosed as pulmonary thromboembolism due to the resemblance in clinical features, such as dyspnea, pleuritic pain, cough and hemoptysis (18). Intimal sarcoma of the pulmonary artery demonstrates an extremely poor outcome, due to delayed intervention and easy recurrence.…”

Insights into etiological factors of pulmonary hypertension in cancer patients

“…The prognosis is poor, but overall survival can improve with complete surgical resection with a potential role for post-surgical chemo and radiotherapy. However, curative surgery is not possible in all patients, and symptomatic benefit can be obtained with other surgical options including pulmonary artery endarterectomy [32].…”